(Professor and Head, Department of Obstetrics and Gynecology, Seth G S Medical College & K E M Hospital, Mumbai, India.)
Port-wine stain, also known as naevus flammeus, is a congenital vascular malformation red, pink or purple in colour. It is usually not affected by pregnancy, though it can affect the pregnancy adversely owing to the presence of associated conditions. A case affected adversely but reversibly in three successive pregnancies is presented. This is the first such case in the world literature.
Port-wine stain is an uncommon disorder encountered in pregnancy. It is a congenital vascular malformation affecting the skin of any part of the body, though it confined to one side in 85% cases. It occurs in 1-3 cases per 1000 births. Since it does not affect most of the cases adversely, most of the patients reach reproductive age and have babies without any mishap. Usually it is not affected adversely by a pregnancy. A case affected adversely but reversibly in three successive pregnancies is presented. This is the first such case in the world literature.
A 26 year old woman, third gravida with two normal deliveries in the past, presented for antenatal registration at our center at 36 weeks of gestation. Her previous children were a girl six years old and a boy 3 years old. She had a port-wine stain on the left side of upper part of the neck, extending on to the adjacent part of the face. It had been present from the time of birth, and had not changed at puberty, nor at any time prior to her first pregnancy. There was no family history of such a condition. She had no history suggestive of any other illness associated with it. Radiological tests done in the past showed no skeletal abnormality of the skull and vertebral column. The stain increased in size and became coarse in texture in both of her previous pregnancies and regressed to pre-pregnancy size and texture after puerperium. In the current pregnancy the stain had increased in size and become coarse in a similar manner, starting from second month of gestation. Her medical, surgical and personal history were not contributory. Her general and systemic examination (including neurological system) findings were normal. The port-wine stain was dull pink to brown in color, situated on the left side of the upper part of her neck and adjacent part of the face. It was coarse in texture. There was no hypertrophy of soft tissues. Her hemogram, coagulation studies, plasma sugar levels, liver and renal function tests and TSH were normal. Her VDRL HIV, HbsAg and anti-HCV tests were negative. Ophthalmological reference was done. Her eyes were normal and there was no glaucoma. Antenatal care was continued and her pregnancy progressed normally. She had a full term normal delivery without any complications like DIC, postpartum hemorrhage or deep vein thrombosis. Her puerperium was uneventful. The port-wine stain regressed totally to her pre-pregnancy state by the end of the puerperium. She was referred to her dermatologist for cosmetic treatment of her port-wine stain, if she so desired.
Figure 2. Port-wine stain after puerperium.
The incidence of a port-wine stain at birth is 0.1-0.3%. It is a congenital vascular malformation affecting the skin containing enlarged capillaries. It can affect any part of the body, though the face and neck seem to be common areas of affection because the other lesions are covered and not seen by most people. It is unilateral in 85% cases, as was seen in our case. Some cases are a part of Sturge Weber syndrome, in which the lesions are seen on the upper part of the face and are associated with similar vascular malformations in the brain, mental retardation, convulsions, unilateral weakness of the body and/or other neurological features.[2,3] If the eyelids are involved, the patient may have glaucoma. The vertebral column may be affected too. Our patient did not have any of these complicating conditions.
Klippel-Trenaunay syndrome is a rare condition in which there is a triad of an extensive port-wine stain, hypertrophy of soft tissue or bone, and venous and/or lymphatic malformation of an extremity. The complications of the syndrome can get exacerbated in pregnancy.[The Klippel-Trenaunay syndrome: clinical, radiological and haemodynamic features and management. Baskerville PA, Ackroyd JS, Lea Thomas M, Browse NL. Br J Surg. 1985 Mar; 72(3):232-6.
Gianlupi A, Harper RW, Dwyre DM, Marelich GP. Recurrent pulmonary embolism associated with Klippel-Trenaunay-Weber syndrome. Chest. 1999 Apr; 115(4):1199-201.] Localized intravascular coagulation may occur with large port-wine stains due to entrapment of platelets and lartge amounts of blood.[4,5] Disseminated intravascular coagulopathy can occur in this syndrome too, which would be of importance to a pregnant woman. Development of a pyogenic granuloma is another complication of this syndrome. Our patient did not have any features of this syndrome.
Pulsed dye laser therapy is the first line of treatment offered for cosmetic treatment of port-wine stain. Its results are quite satisfactory.[8,9] Associated other conditions require treatment by respective specialists.
What is unique in the case presented is that the skin lesion increased in size and became coarser during three consecutive pregnancies and regressed totally to the pre-pregnant state after puerperium. This would suggest an estrogen-progesterone dependence of the lesion in a woman. It would be interesting to see what happens to the lesion if she takes combination contraceptive pills (it is expected to behave as in a pregnancy) and after she develops menopause (there may not be any change, as the lesion existed prior to puberty without hormone levels as during the reproductive age).
- Up to Date: Vascular lesions and congenital naevi in the newborn [online]. 2005. Available from: http://www.utdol.com/application/topic.asp?file=ped_derm/4439&type=P&selectedTitle=3~5
- S. J. Garro and W. T. Bradshaw, “Sturge-Weber syndrome: a case study,” Advances in Neonatal Care, vol. 14, no. 2, pp. 96–102, 2014.
- Enjolras O, Riche MC, Merland JJ. Facial port-wine stains and Sturge-Weber syndrome. Pediatrics 1985; 76:48-51.
- D'Amico JA, Hoffman GC, Dyment PG. Klippel-Trénaunay syndrome associated with chronic disseminated intravascular coagulation and massive osteolysis. Cleve Clin Q. 1977 Winter; 44(4):181-8.
- Stein SR, Perlow JH, Sawai SK. Klippel-Trenaunay-type syndrome in pregnancy. Obstet Gynecol Surv. 2006 Mar; 61(3):194-206.
- Fishman A, Paldi E. Klippel-Trenaunay syndrome with complications during pregnancy. Harefuah. 1989 Feb 1; 116(3):147-8.
- Rodins K, Gramp D, James D, Kumar S. Pyogenic granuloma, port-wine stain and pregnancy, Australasian Journal of Dermatology, 2011;52(4):e8–e10, 2011.
- Vinciullo C. Pulsed dye laser treatment of port-wine stains: a review of patients treated in Western Australia. MJA 1996; 164: 333-336.
- Brightman LA, Geronemus RG, and K. K. Reddy, “Laser treatment of port-wine stains,” Clinical, Cosmetic and Investigational Dermatology, vol. 8, pp. 27–33, 2015.
Parulekar SV. Port Wine Stain In Pregnancy. JPGO 2019. Vol 6 No. 3. Available from: https://www.jpgo.org/2019/03/port-wine-stain-in-pregnancy.html